Overview

Myelodysplastic syndrome (MDS) is a heterogeneous group of clonal haematologic disorders characterised by ineffective hematopoiesis and, usually, a hypercellular bone marrow.

The cells of the affected lineages are unable to mature normally, and this impaired ability to undergo normal differentiation and maturation results in peripheral blood cytopenias.

MDS are considered preleukemic diseases, although not all cases terminate in acute myeloid leukemia (AML). These syndromes are usually diagnosed in older adults and their natural history ranges from an occasionally rapid progression into AML, to a chronic course that can last for years.

MDS is the most common haematological malignancy in people aged > 70 years. MDS is rare in children and adolescents and often develops in the context of a predisposing genetic condition.

Patients usually present with anaemia, neutropenia, thrombocytopenia, or a combination of these cytopenias. A bone marrow is usually required for diagnosis although this may not be appropriate in older patients with comorbidities.

The WHO classification for MDS is widely accepted and has recently been updated (2016).

References are given throughout the chapter and the following articles may be of interest:

Arber, D. A., et al. (2016). "The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia." Blood 127(20): 2391-2405.

Fenaux, P. and L. Adès (2013). "How we treat lower-risk myelodysplastic syndromes." Blood 121(21): 4280-4286.

Hoffbrand, A. V., et al. (2012). "How I treat transfusional iron overload." Blood 120(18): 3657-3669.

Killick S.B., (2017). “Iron chelation therapy in low risk myelodysplastic syndrome.” British Journal of Haematology 177(3): 375-387.

Killick, S. B., et al. (2014). "Guidelines for the diagnosis and management of adult myelodysplastic syndromes." British Journal of Haematology 164(4): 503-525.

Malcovati, L., et al. (2013). "Diagnosis and treatment of primary myelodysplastic syndromes in adults: recommendations from the European LeukemiaNet." Blood 122(17): 2943-2964.


About this Canterbury DHB document (4952):
Document Owner:	Emma-Jane McDonald (see Who's Who)
Last Reviewed:	November 2019
Next Review:	November 2021
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Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer The Haematology Department Protocols and Guidelines (the Red Book) are intended as a guide for registered health professionals to communicate guidelines and share best practices with supporting health professionals within New Zealand district health boards. They are not intended to be provided to or used as a reference for patients or non-registered health professionals. All health professionals must exercise their own clinical judgement and use pertinent clinical data when treating patients. The authors and editors of this publication have checked with sources believed to be reliable in their effort to provide information that is complete and in accord with the standards accepted at the time of publication. However, in view of the possibility of human error or changes in medical science, neither the authors, editors, publisher, nor any other party who has been involved in preparing or publishing this work warrants that the information contained in it is accurate or complete in every respect. They therefore have no liability (whether in tort (including negligence) or otherwise) for any loss or damage arising out of any reliance on this information. This publication has been compiled by the staff and contractors of Canterbury District Health Board (CDHB) and as such CDHB has copyright for this book. Amendments must be authorised in accordance with the documented process. No release of part of, or all of, this publication to any other party or organisation is permissible without the prior approval of the designated document owner.
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Topic Code: 4952